MKSAP Pulm III

Question 1

Why people test for vasoreactivity with nitric oxide in PAH patients?

Answer 1

To identify those who may respond to CCVs

Question 2

What is Pirfenidone?

Answer 2

antifibrotic agent indicated for the treatment of idiopathic pulmonary fibrosis.

Question 3

What is bosentan?

Answer 3

On of many oral pulm vasoactive drugs in pts who do not respond to nitric oxide (it is a endothelin receptor antagonist)

Question 4

Normal pulmonary artery systolic pressure

Answer 4

18 to 25 mm Hg

Question 5

Masses in the anterior mediastinum are usually recalled as the “terrible T’s” (4, granted one starts with “terrible” lol)

Answer 5

1. thymoma (assoc with Myasthenia gravis)
2. teratoma/germ cell tumor
3. “terrible” lymphoma
4. thyroid

Question 6

In patients with severe COPD and frequent exacerbations, chronic ____ has been shown to decrease COPD exacerbations.

Answer 6

macrolide therapy

Question 7

What is Lymphangioleiomyomatosis?

Answer 7

a multisystem disease that almost exclusively affects young women. Pulmonary complications are prominent and include diffuse pulmonary cysts, pneumothorax, chylous pleural effusions, and obstructive airways disease. Lymphangioleiomyomatosis would be very unlikely in a patient with an unremarkable chest radiograph.

Question 8

What is Lymphoid interstitial pneumonia?

Answer 8

an interstitial lung disease characterized by lymphocytic infiltration of the pulmonary interstitium. It is observed in patients with Sjögren’s syndrome and viral infections, especially HIV. Patients with lymphoid interstitial pneumonia often have crackles on the pulmonary examination, interstitial infiltrates on chest radiography, and decreased lung volumes and diffusing capacity on pulmonary function testing.

Question 9

What is organizing pneumonia?

Answer 9

patchy process that involves proliferation of granulation tissue within alveolar ducts, alveolar spaces, and surrounding areas of chronic inflammation. There are many known causes of this pattern, including acute infections and autoimmune disorders such as rheumatoid arthritis. The term cryptogenic organizing pneumonia (COP) is reserved for individuals who have this pattern but do not have a clear associated cause. Patients with COP will typically present with symptoms during 6 to 8 weeks that mimic community-acquired pneumonia. Evaluation typically demonstrates bilateral diffuse alveolar opacities on chest radiograph with normal lung volumes.

Question 10

_____ is commonly associated with connective tissue diseases, such as limited cutaneous systemic sclerosis.

Answer 10

Pulmonary arterial hypertension

Question 11

Recurrence prevention with pleurodesis is recommended after the ____ occurrence of secondary spontaneous pneumothorax.

Answer 11

first, because likelihood of recurrence is very high

Question 12

For adults with a hypertensive emergency and without a compelling condition (such as aortic dissection) systolic blood pressure should be reduced by no more than ____ within the first hour; then, if stable, to _____ within the next 2 to 6 hours; and then cautiously to normal during the following 24 to 48 hours.

Answer 12

25%; 160mmHg

Question 13

Severe cases presents as multiorgan failure with coma, seizures, and cardiovascular symptoms, including hypotension, bradycardia, heart block, and ventricular arrhythmias. Early manifestations are nonspecific. Diagnostic clues include lactic acidosis and inappropriately elevated central venous oxyhemoglobin saturation, which manifests as bright red venous blood. Associated with house fires.

Answer 13

Cyanide poisoning

Question 14

What is sodium thiosulfate used for?

Answer 14

Cyanide toxicity but second line because it works slower

Question 15

What is methylene blue used for?

Answer 15

toxic levels of methemoglobin, usually 20% to 30% or higher